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Specialty:

Pediatrics

Secondary Specialty:

Nephrology (Kidneys)
Pediatric Nephrology

Board Certifications:

  • Pediatric Nephrology
  • Pediatrics

Education:

MD, Academia Medica Gedanensis Gdansk, Poland

Residency:

Saint Vincent's Hospital and Medical Center of New
New York, NY
Saint Vincent's Hospital and Medical Center of New
New York, NY

Fellowships:

Montefiore Medical Center The University Hospital for the Albert Einstein College of Medicine
Bronx, NY

Affiliations:

Children's Hospital of Pittsburgh of UPMC

Languages:

Polish

Agnieszka Swiatecka-Urban, MD

Doctor

Division of Pediatric Nephrology, Children's Hospital of Pittsburgh of UPMC
Associate Professor of Pediatrics, University of Pittsburgh School of Medicine
Associate Professor of Cell Biology, University of Pittsburgh School of Medicine

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Biography

Research Interests

The role of Rab GTPases in regulating the plasma membrane half-life of CFTR.
The cystic fibrosis transmembrane conductance regulator (CFTR) is a cAMP-activated chloride channel that is expressed in the apical plasma membrane in many epithelial tissues. Cystic fibrosis (CF) is caused by mutations in the CFTR gene and is one of the most common lethal inherited disorders in Caucasians (1 in 3,200 live births affected in the US). The most common CF mutation, ∆F508 causes: (1) retention of CFTR in the endoplasmic reticulum (ER), (2) reduced open probability of the CFTR chloride channel, and (3) decreased plasma membrane half-life of CFTR. Thus, correction of the defects caused by the ∆F508 mutation will require a combined therapy that includes increased exit from the ER, increased chloride channel activity, and increased plasma membrane half-life. Of these points, particularly little is known about the mechanisms that regulate the plasma membrane half-life of ∆508-CFTR. The biochemical plasma membrane half-life of CFTR is at least in part determined by the relative rates of CFTR endocytosis, recycling, and degradation. Rab proteins, a large family of small GTPases, facilitate a variety of trafficking events, including endocytosis and sorting to a recycling or a degradative pathway. The role of Rab GTPases in regulating CFTR trafficking is not completely understood. In particular, little is known about the role of Rab GTPases in sorting CFTR between the recycling and degradative compartments. Thus, my long-term goal is to elucidate these mechanisms in order to develop a strategy to increase the expression of ∆F508-CFTR in the plasma membrane.

The role of nephrin trafficking in maintaining the dynamic structure of the glomerular slit diaphragm (pathomechanisms of nephrotic syndrome).
Nephrin, a cell adhesion molecule of the immunoglobulin (IgCAM) superfamily is a transmembrane protein expressed in the podocyte foot processes. Podocytes, the visceral glomerular epithelial cells, form a close-fitting network of interdigitating cellular extensions – foot processes – linked by modified tight junctions, called slit diaphragms. The slit diaphragm is a dynamic structure and a crucial component of the filtration barrier that plays a major role in the size selectivity, preventing passage of proteins and other molecules larger than albumin into the glomerular filtrate, and ultimately into the urine. Nephrotic syndrome is a very common kidney disease in children and adults. The pathomechanism of nephrotic syndrome is not well understood and remains one of the most important questions in nephrology today. The characteristic ultrastructural changes observed during the development of nephrotic syndrome – retraction and effacement of the podocyte foot processes – lead to disruption of the slit diaphragm and proteinuria. These morphological changes are closely associated with altered expression of nephrin. Mutations in the nephrin gene are responsible for many forms of congenital nephrotic syndrome and downregulation of nephrin expression correlates with the degree of proteinuria in diabetic nephropathy. Although nephrin is critically important for the integrity of the slit diaphragm, the exact mechanisms are not well understood. In particular, very little is known about the role of nephrin trafficking in maintaining the dynamic structure of the filtration barrier. Thus, my long-term goal is to elucidate these mechanisms in order to develop strategies to prevent nephrotic syndrome.

  • Cell Biology of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR)
  • Regulation of the Endocytic Trafficking of CFTR
  • Regulation of Endocytic Trafficking of Nephrin

View Dr. Swiatecka-Urban's full list of publications from PubMed.

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